Abstract
Introduction
In this report we describe an aged female patient who had a 7-year history of short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), consisting of short-lasting, right-sided attacks of severe periorbital headaches, with symptoms of ipsilateral autonomic dysfunction. The attacks were resistant to various medical treatments, including indomethacin, but ceased abruptly after a left-hemispheric ischaemic stroke in the territory of the medial cerebral artery. The patient remained attack-free during a follow-up of 1 year.
Case report
This woman, at present 81 years old, had attacks of pain behind the right eye, which would last several minutes and occurred up to seven times per day. The pain was associated with ipsilateral tearing of the eye, conjunctival injection, eyelid oedema and rhinorrhoea, but not with nausea, vomiting or photo- and phonophobia. During these attacks she would press with her hand on her right eye, while rocking back and forth. Touching her ipsilateral eyebrow would sometimes trigger the attacks. She would also have periods of continuous periorbital pain superimposed by shoots of pain paroxysms lasting seconds to minutes. The attacks started 7 years ago, without any clear precipitants. A patient diary showed two to seven attacks per day, with a duration of up to 5 min. She would be attack-free for at most several consecutive days.
Neurological examination revealed a slight ptosis on the right side, but no further abnormalities. Blink reflex was normal. Computed tomography (CT) scans of the brain and the orbit and two magnetic resonance imaging (MRI) scans of the brain before and after administration of gadolinium (with an interval of 2 years) were normal. Ophthalmological examination also revealed no abnormalities.
The attack frequency would fluctuate spontaneously without any apparent influence of a variety of treatments, including simple analgesics, carbamazepine (max. 800 mg/day), phenytoin (max. 300 mg twice per day), valproate (max. 1000 mg/day), baclofen (max. 60 mg/day), indomethacin (max. 100 mg/day), verapamil (max. 240 mg/day) and various combinations of these.
At the age of 80, the patient suffered from an ischaemic stroke in the territory of the left medial cerebral artery, causing right-sided hemiplegia, central facial paresis and complete aphasia. A CT scan showed a left medial cerebral artery infarct and a hyperdense medial cerebral artery, indicating thrombosis (Fig. 1). The attacks ceased immediately and verapamil and carbamazepine, which she was taking at that time, although their efficacy was doubtful, could be subsequently stopped. Whilst a full evaluation was complicated by the aphasia, there were no signs of paroxysmal restlessness or pain, nor any autonomic symptoms. She has remained attack-free until the present day, 1 year later.
Computed tomography scan of the brain showing a hypodense lesion in the left medial cerebral artery territory (a). The hyperdense medial cerebral artery indicates thrombosis (b).
Discussion
This patient probably suffered from SUNCT, in which patients usually have frequent attacks of brief unilateral, orbital pain accompanied by ipsilateral autonomic symptoms. The attack frequency may fluctuate from nil up to 100 per day and may be triggered by touching the face (1, 2). SUNCT predominantly occurs in males, has a relatively late age at onset and typically does not respond to medication (3). In our patient, trigeminal neuralgia of the first branch seems unlikely, because these attacks are usually of shorter duration and are very rarely associated with the full range of autonomic symptoms (4, 5). Chronic paroxysmal hemicrania (CPH) also seems improbable, as the attacks were too short-lasting, could be triggered by touching the face, and did not respond to indomethacin (6). Although the indomethacin dosage was not increased above 100 mg/day, which is usually adequate, we considered this maximal for this elderly patient, taking into account the possible side-effects.
The pathophysiology of SUNCT is unknown. Neuroimaging is usually uneventful; however, some patients with SUNCT have been described in association with extracerebral, ipsilateral lesions (7–10). Here we report for the first time the temporal association between the occurrence of a structural lesion and disappearance of attacks. We can suggest no mechanism to explain this.
It might be possible that this patient suffered from agnosia for pain, which has been described in association with lesions in the (dominant) parietal lobe, and therefore did not experience the pain paroxysms. However, the absent signs of paroxysmal restlessness and autonomic symptoms after the stroke make this very unlikely. A spontaneous remission is possible, but seems improbable in this patient, as she had not had long-lasting attack-free periods.
A recent publication reports two SUNCT patients with continuing attacks after several neurosurgical interventions of the peripheral trigeminal root, suggesting that the causative lesion in SUNCT lies within the central nervous system (11). In accordance with a possible central mechanism is another report of a SUNCT patient with attacks occurring shortly after a presumed ipsilateral brainstem infarction (12). Ipsilateral activation of the hypothalamic grey matter in a functional MRI study further confirms a central mechanism (13). Interestingly, the peripheral and central lesions reported in these case descriptions are all ipsilateral to the SUNCT attacks they possibly engendered, whereas the lesion described in our patient that apparently has led to disappearance of the attacks occurred contralaterally. The relation of these lesions to the side of occurrence of attacks or the cessation of attacks is difficult to explain.